Unlike extrahepatic bile duct stones, intrahepatic bile duct stones are primary stones, mainly bile pigment stones containing bacteria. The causes are mainly related to biliary tract infections, biliary stasis, biliary parasites, and abnormal bile duct anatomy. Intrahepatic bile duct stones do not always cause clinical symptoms and are sometimes detected during a health examination. Isolated intrahepatic bile duct stones should be distinguished from intrahepatic calcified foci, which are sometimes difficult to distinguish. The symptoms of intrahepatic bile duct stones are mainly cholangitis, which may present with high fever, chills, abdominal pain and jaundice, and in severe cases, bile duct stricture and liver atrophy. For symptomatic intrahepatic bile duct stones, in principle, surgery or interventional treatment should be actively performed, depending on the size and location of the stone and the condition of the liver. Intrahepatic bile duct stones can sometimes be complicated to manage and may even require multiple, staged surgical procedures. It is also important to note that intrahepatic bile duct stones may cause cancer and proper treatment is very important.